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Related Concept Videos

Autoimmune Disorders01:29

Autoimmune Disorders

792
Autoimmune diseases are a group of disorders in which the body's immune system mistakenly attacks its own cells, tissues, and organs. This results from an overactive immune response against substances and tissues normally present in the body. Let's delve into the concept and mechanism of autoimmune diseases from an immune system point of view, explore different causes and examples of such diseases, and discuss potential solutions.
Concept and Mechanism of Autoimmune Diseases
The immune...
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Objective sleep profile in LGI1/CASPR2 autoimmunity.

Michelle F Devine1,2,3,4, John C Feemster1,5, Elizabeth A Lieske5

  • 1Mayo Clinic Center for Sleep Medicine, Mayo Clinic and Foundation, Rochester, MN, USA.

Sleep
|December 25, 2021
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Summary
This summary is machine-generated.

Leucine-rich, glioma inactivated protein 1-IgG (LGI1) and contactin-associated protein 2-IgG (CASPR2) autoimmunity frequently causes REM sleep behavior disorder (RBD) and other sleep issues. Polysomnography reveals objective markers, and immunotherapy can improve these sleep disturbances.

Keywords:
CASPR2LGI1Morvan syndromeREM behavioral disorderautoimmune sleep

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Area of Science:

  • Neurology
  • Sleep Medicine
  • Immunology

Background:

  • Leucine-rich, glioma inactivated protein 1-IgG (LGI1) and contactin-associated protein 2-IgG (CASPR2) autoimmunity are increasingly recognized neurological disorders.
  • Sleep disturbances, including REM sleep behavior disorder (RBD), are common in LGI1/CASPR2 autoimmunity, but detailed polysomnographic analyses are limited.

Purpose of the Study:

  • To characterize the clinical presentations of sleep disturbances in LGI1/CASPR2-IgG seropositive patients.
  • To analyze polysomnographic manifestations, focusing on quantitative REM sleep without atonia (RSWA).

Main Methods:

  • Retrospective analysis of clinical and polysomnographic data from LGI1+/CASPR2+ patients and age-sex matched controls.
  • Quantitative analysis of combined submentalis and anterior tibialis (SM + AT) RSWA as the primary outcome.
  • Statistical comparison using Wilcoxon rank-sum and chi-square tests.

Main Results:

  • Seven out of 11 patients (63.6%) exhibited Morvan syndrome sleep features. Common disturbances included dream enactment behavior (DEB), insomnia, and sleep apnea.
  • LGI1+/CASPR2+ patients showed increased N1 sleep (p = .02), decreased REM sleep (p = .001), and significantly higher SM + AT RSWA (p < .001).
  • Eighty-nine percent of LGI1+ patients exceeded RBD RSWA thresholds, with RSWA greater in anterior tibialis than submentalis muscles.

Conclusions:

  • LGI1/CASPR2-IgG autoimmunity is strongly associated with prominent dream enactment, insomnia, increased RSWA, sleep apnea, and shallower sleep.
  • Polysomnography offers objective disease markers for LGI1+/CASPR2+ autoimmunity.
  • Immunotherapy demonstrates potential benefits for associated sleep disturbances in these patients.